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Open Journal of Ophthalmology, 2012, 2, 71-77

http://dx.doi.org/10.4236/ojoph.2012.23015 Published Online August 2012 (http://www.SciRP.org/journal/ojoph)

Childhood Glaucoma: An Overview

Parul Singh, Yogesh Kumar, Manoj Tyagi, Krishna Kuldeep, Parmeshwari Das Sharma
Department of Ophthalmology, Veer Chandra Singh Garhwali Government Medical Sciences and Research Institute, Srinagar, India.
Email: parulophtha@gmail.com
Received March 16th, 2012; revised April 27th, 2012; accepted May 28th, 2012

ABSTRACT
Several types of childhood glaucoma exist, and the terminology is based on the time of onset of disease and its potential
cause. Though childhood glaucoma occurs less commonly than adults but can lead to permanent visual damage due to
amblyopia, optic neuropathy or refractive error. A detailed evaluation should be done to establish diagnosis. Medical
therapy has a limited role and surgery remains main modality for treatment. Childhood glaucoma is a treatable disease,
if early diagnosis is established and therapeutic intervention done in time. In children with low vision efforts should be
there to maintain residual vision and visual rehabilitation with low vision aids should be done.
Keywords: Childhood Glaucoma; Goniotomy; Trabeculotomy

1. Introduction
Glaucoma is less common in children than in adults. Although glaucoma can lead to permanent visual damage at
any age, the consequences of the disease are more often
severe in children due to additional damage that can
happen to the developing visual system. Associated amblyopia and secondary refractive errors are common.
Primary congenital glaucoma (PCG) is hereditary childhood glaucoma secondary to abnormal development of
the filtration angle. Nonetheless, most ophthalmologists
usually encounter the wide range of secondary forms of
glaucoma in this age group. Glaucoma surgery has drastically improved the visual prognosis of children afflicted
with glaucoma. However, late diagnosis of the condition
can result in permanent and severe visual morbidity. Its
affliction of young children makes glaucoma control a
life-long goal requiring motivation and perseverance by
patients, their parents and doctors. This article is intended to provide an overview of the disease at genetic
levels, newer technological tools assisting in diagnosis,
IOP lowering medications and refined surgical techniques.

Relating to development pattern

1) Developmental glaucoma: This term broadly encompasses all glaucomas resulting from abnormal development of the aqueous outflow system. This may or
may not be associated with systemic anomaly;
2) Primary developmental glaucoma: This is the glaucoma resulting from maldevelopment of aqueous outflow
system;
3) Secondary developmental glaucoma: This refers to
glaucoma resulting from damage to aqueous outflow
system due to maldevelopment of some other portion of
the eye, e.g. eye with microspherophakia or dislocated
lens.
Relating to anatomy
Hoskins classified developmental glaucoma anatomically by the structures involved:
1) Trabecular meshwork: Trabeculodysgenesis;
2) Iris and trabecular meshwork: Iridotrabeculodysgenesis;
3) Cornea and trabecular meshwork: Corneotrabeculodysgenesis [1].

1.1. Definition and Terminology

1.2. Classification of Congenital and Infantile

Glaucoma

Relating to age of onset

1) Congenital glaucoma: The glaucoma that exists at
birth, and usually before birth;
2) Infantile glaucoma: Glaucoma that occurs from
birth until 3 years of age;
3) Juvenile glaucoma: Occurs after the age of three to
teenage years.

Primary congemital glaucoma (Trabeculodysgenesis)

Secondary congenital glaucoma
1) Iridocorneotrabeculodysgenesis
a) Riegers anamoly;
b) Axenfeld anamoly;
c) Peters anamoly.
2) Iridotrabeculodysgenesis

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Childhood Glaucoma: An Overview

a) Stromal defects: Hypoplasia, hyperplasia;

b) Anomalous iris vessels: Persistence of tunica vasculosa lenti;
c) Structural anamolies: Holes, coloboma, aniridia;
Acquired glaucomas
1) Aphakia;
2) Corticosteroid induced;
3) Retinoblastoma;
4) Traumatic;
5) Uveitis.

2. Epidemiology
Primary congenital glaucoma is hereditary with a variable incidence in different populations, but an overall
occurrence of 1 in 10,000 births is seen [2]. A greater
incidence occurs in populations with higher rates of consanguinity [3,4]. Boys are more commonly affected in
United States and Europe with boys to girls ratio of 3:2.
Whereas, in Japan more girls are seen having this condition [5,6]. The majority (about 75%) of PCG cases are
bilateral and asymmetric expression should be suspected
in clinically apparent unilateral cases. More than 80%
patients present within the first year of life, with 25%
diagnosed in the neonatal period and 60% within the first
6 months of life [7].

3. Genetics
The majority of PCG cases are sporadic but 10% - 40%
are familial with frequent association with consanguinity
[8]. In the most familial cases, transmission is autosomal
recessive with variable expression and penetrance of
40% - 100% [8].
Genetic heterogenicity of PCG confirmed by linkage
studies explain discrepancies like unequal sex distribution, lower than expected number of affected siblings in
familial cases, and transmission of the disease to successsive generations [8]. Three loci for PCG have been found
[8-10]. The initial locus on chromosome 2p21 (GLC3A)
was described in 1995 by Sarfarazi et al. who identified
significant genetic linkage to this region in 11 of 17 turkish families [8,11]. Genetic heterogenicity was confirmed
when a second locus on chromosome 1p36 (GLC3B) was
found [9]. Genetic family analysis identified a third locus
GLC3C on chromosome 14q24.3 [10]. Although three
chromosomal loci have been linked to PCG, only
CYP1B1 in locus GLC3A has been identified [12]. Mutations in these genes have been described as the predominant cause of PCG in Turkish and Saudi arabian
families [13]. It has been reported that 87% of familial
and 27% sporadic case are due to mutation in this gene
[8]. Approximately 45 mutations of this gene have been
identified and include deletion, insertion, point mutation,
mis-sense, non-sense, frameshift and terminator mutation
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[14]. From all mutations studied, frameshift and R390C

homozygous mutations were associated with severe phenoltype and very poor prognosis. Primary juvenile glaucoma can be inherited in an autosomal dominant fashion.
A mutation in the chromosome 1q23-25 region has been
linked to this disease [15,16]. Genetic studies have also
been conducted on few other glaucomas. In Axenfeld,
Rieger anomaly, the gene has been mapped to chromosome 6p25 [17]. In aniridia, the most common inheritance pattern is autosomal dominant. The genetic locus
for aniridia has been established as the PAX6 gene, located on 11p13 [18].

4. Pathogenesis
The exact mechanism involved in pathophysiology of
primary infantile glaucoma is not known. One theory
said that a pathologic membrane (known as Barkans
membrane) [19] covered and blocked the trabecular
meshwork in primary infantile glaucoma. Clinical and
histopathologic observations of the anterior chamber in
infantile glaucoma have revealed that the anatomic relationship between the iris, trabecular meshwork and
ciliary body are immature. The principal defect in primary infantile glaucoma is a failure of one or more steps
in the normal development of the anterior chamber angle.
As the genes associated with primary infantile glaucoma are characterized further and the physiological or
developmental role of the proteins they encode become
better understood; the molecular, cellular and embryological pathophysiology of this rare disorder will become
clear [12].
Among the secondary glaucomas of childhood, the
underlying pathophysiology is as varied as that in adults.
Occurrence at or shortly after birth indicates a profound
developmental abnormality of anterior chamber angle
whereas, manifestation later usually suggests a different
process.
Secondary open angle also occur in young children.
Both corticosteroid-induced and chronic uveitic glaucomas are described [20]. It is difficult to classify the underlying cause of glaucoma, that frequently follows pediatric cataract extraction. Walton examined 65 children
in whom, pre-operative gonioscopy revealed no consistent angle defect but post-operative gonioscopy revealed
filtration angle deformity [21]. Retained lens material
was one of the risk factor known another cause may be
presence of small cornea.

5. Presentation
The signs and symptoms of PCG are variable dependent
on childs age and severity of glaucoma; and secondary
corneal abnormalities. PCG is characterized by clinical
triad of epiphora, blepharospasm and photophobia, but
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these symptoms are often missed until the more alarming

corneal edema becomes apparent. The corneal edema,
may be subtle, especially in bilateral cases; or profound
with enlarged corneal diameter and globe, breaks in Descemets membrane (Haabs striae) and sometimes even
acute hydrops.
Myopia is typical finding in infantile glaucoma. In
older children, astigmatism and progressive axial myopia
cause symptomatic decreased uncorrected visual acuity
and refractive amblyopia.
Optic neuropathy is the most concerning consequence
from glaucoma, because the neuropathy is irreversible
once axonal death has occurred.
While cupping of the optic nerve in glaucoma is generally a gradual process in older children and adults, it
can occur rapidly in infants. Reversibility of the cupping
with normalization of the intra-ocular pressure (IOP) in
young children occurs due to suspected relative immaturity and elasticity of lamina cribrosa. Less common presenting signs in infantile glaucoma include conjunctivitis,
blepharitis and cellulitis.

If the diagnosis of glaucoma is confirmed or strongly

suspected based on clinical examination, an examination
under anaesthesia is required. Unfortunately, most anesthetic agents and sedatives have lowering effect on IOP
[24]. Tonometry should be best performed as soon as
possible after induction of anesthesia. Corneal diameters
are measured with a millimeter ruler or caliper and recorded. Detailed hand slit lamp examination of anterior
segment is followed by gonioscopy and fundus examination.
Visual field defects are similar to those seen in adults
with initial predilection for loss in arcuate areas, but
child should be able to follow directions for field examination (perimetry). Although the technology has been
around and algorithm is getting better and better, it is still
difficult to apply them in children as they may not follow
instructions or may not cooperate. Optical coherence
tomography may be a better option and much easier as
compared to visual field examination in young and noncooperative patients.

7. Differential Diagnosis
6. Diagnosis and Ancillary Testing
It is important to do a complete ophthalmic examination
in a child suspected of glaucoma. This includes IOP
measurement, gonioscopy, optic nerve head examination
and refraction. Check for the childs ability to fix and
follow; and for the presence of nystagmus. Examination
of the cornea is crucial with respect to size and clarity of
the cornea and the presence of Haabs striae.
This examination can be done in clinic. With some
practice, IOP can be measured in a conscious, swaddled
infant using perkins tonometer or tonopen. Usually IOP
in infants with normal eyes is in the range of 11 - 14 mm
Hg using these devices. The measurement of IOP greater
than 20 mmHg in a calm, resting infant is suspicious for
glaucoma when other signs and symptoms also suggest
the disease. Measurements of IOP undertaken while a
child cries and resists efforts to hold the eye open are
invalid.
Examination of the optic nerve is attempted, because
obvious cupping confirms the diagnosis. Shaffer and
Hetherington noted a cup to disc ratio (C/D ratio) greater
than 0.3 in 68% of 126 eyes affected by primary infantile
glaucoma [22], whereas C/D ratio greater than 0.3 was
found in less than 2.6% of newborns with normal eyes
[23]. A Koeppe infant diagnostic lens offers good visualization of disc using direct ophthalmoscope. Gonioscopy can also be performed with it even in a conscious
infant. In the normal newborn eye, the iris usually inserts
posterior to the scleral spur. In PCG, the iris commonly
inserts anteriorly directly into the trabecular meshwork.
This iris insertion is most commonly flat, although concave insertion may be rarely seen.
Copyright 2012 SciRes.

Signs and symptoms

Condition

Tearing, discharge,
conjunctival injection

Nasolacrimal duct
obstruction infections
Allergic conjunctivitis

Cloudy cornea, loss of

corneal lusture, Haabs
striae

Corneal dystrophy e.g.

CHED, PPMD Forcep
trauma Cysticercosis
Sclerocornea
Mucopolysaccharidosis
I, Is, II, III infectious keratitis

Enlarged cornea,
apparent asymmetry
of globe size

Primary megalocornea
Unilateral high myopia
Proptosis lid retraction
Contralateral microphthalmos
Enopthalmos ptosis

Photophobia,
blepharospasm

Uveitis corneal infection,

abrasion or dystrophy
Retinal cone dystrophy

High myopia

Pathologic myopia
Vitreo-retinal degeneration
e.g. Sticklers syndrome

Enlarged cup to
disc ratio

Physiologic optic nerve

cupping optic nerve
atrophy or anomaly
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Childhood Glaucoma: An Overview

8. Management
The definitive treatment for primary infantile glaucoma
is surgical. Medical therapy usually provides a supportive role to reduce the IOP temporarily, to clear the cornea, and to facilitate surgical intervention. Laser therapy
has limited role in developmental glaucomas. Primary
surgical treatment is usually with goniotomy or trabeculotomy, although combined trabeculotomy with trabeculectomy may be useful in certain populations with high
risk of failure of goniotomy or trabeculotomy. Refractory
congenital glaucomas may be managed by trabeculectomy
with anti-fibrosis drugs, glaucoma drainage implants and
cyclodestructive procedures (Figure 1).

8.1. Medical Management

Medications do play a limited role in the treatment of
childhood glaucoma. In primary infantile glaucoma,
medications may be used preoperatively to clear the corneal edema or post-operatively if the response to surgery
is borderline and more time is needed to determine if
further surgery is required.

8.1.1. Beta Blockers

They lower IOP by decreasing production of aqueous in
the ciliary body. The drug should be used with extreme
caution in neonates due to the possibility of bronchospasm, apnea and bradycardia. Cardiac abnormalities and
bronchial asthma should be specifically excluded before
its use. Use of 0.25%, rather than 0.5% is recommended
in children, in order to reduce its side-effects. In 100 eyes
with childhood glaucoma treated with timolol, 31% patients experienced a reduction in IOP [25].
8.1.2. Alpha-2 Agonists
They also decrease aqueous production but their use in
children is limited because of central nervous system
depression. In 30 patients with mean age of 10 years,
brimodine treatment was associated with a mean reduction of IOP by 7% [26]. Two young children were transiently unarousable and five other children experienced
extreme fatigue [26]. In another study involving 23 patients with mean age of 8 years, 18% had systemic adverse effects that necessitated stopping of the drug [27].

Congenital Glaucoma Suspect

Corneal Diameter measurement

Tonometry
Gonioscopy
Ophthalmoscopy
Slit Lamp examination
Visual field analysis (if possible)

Normal Examination

Congenital Glaucoma

Follow-up

Goniotomy
Trabeculotomy
Trabeculectomy
Combined trabeculotomy and trabeculectomy

Unsuccessful
results

Successful
results

Follow-up

Filtration surgery with antifibrotic drugs

Glaucoma drainage implants
Cyclodestructive procedures

Figure 1. Flowchart of management of congenital glaucoma.

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Childhood Glaucoma: An Overview

8.1.3. Carbonic Anhydrase Inhibitors (CAIs)

They suppress aqueous production and are available as
either an oral formulation or as topical drop. The oral
formulation may be more effective in IOP lowering but
also produces more side-effects of diarrhea, lethargy,
poor appetite and metabolic acidosis [28]. The mean was
reduced by 36% and 27% compared with baseline, after
treatment with acetazolamide and topical dorzolamide,
respectively. All eyes showed an increase in IOP when
switched from acetazolamide to dorzolamide with mean
increase of 3.7mmHg [29]. Topical CAI can be expected
to have additive benefit when used in conjunction with
beta-blockers [30].
8.1.4. Prostaglandins Analogues
These drugs reduce IOP by enhancing uveo-scleral outflow [31]. Their side-effects include conjunctival hyperemia, iris and skin pigmentation and accelerated eyelash growth. High non-response rate has been reported in
children with little IOP lowering in responders [32].
8.1.5. Miotics
They are ineffective for PCG, but can be used preoperatively before angle surgery.

8.2. Surgical Treatment

Early surgical intervention is of prime importance in the
management of patients with PCG. Either goniotomy or
trabeculotomy is the procedure of choice; goniotomy
requires clear cornea while trabeculotomy may be performed if cornea is hazy or opaque. When greater
chances of failure are present, trabeculotomy may be
combined with trabeculectomy.
8.2.1. Goniotomy
A blade is inserted through the peripheral cornea 180
across from meshwork to be incised (usually the nasal or
the temporal portions) and is then guided across the anterior chamber into the chamber angle. With the aid of gonioscopy lens, the trabecular meshwork is visualized, and
the blade is used to make a linear incision through the
meshwork for approximately one third of the circumference of eye. The reported rate of success of goniotomy in
infantile glaucoma is 80% [33]. Endoscopic goniotomy
has been successfully performed utilizing a co-axial ocular endoscope in presence of corneal opacification, which
prevented standard goniotomy procedure [34].
8.2.2. Trabeculotomy
A scleral flap is created over the area of network to be
incised. Through this flap a dissection into schlemms
canal is created. A trabeculotome is inserted into the canal and the meshwork is then opened, as instrument is
rotated into the anterior chamber. Considering congenital
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glaucoma of all grades of severity, trabeculotomy controls IOP in over 90% of eyes. The efficacy of this operation compared favorably with goniotomy. The results of
trabeculotomy and goniotomy for infantile glaucoma
were compared and found equally effective and safe [35].
The significant advantage of trabeculotomy for those
cases with a cloudy cornea limiting visualization of the
angle was described [36]. A newer trabeculotomy technique with protein suture passed 360 through Schlemms
canal has been mentioned [37].
8.2.3. Trabeculectomy
A trabeculectomy involves creating a full thickness
opening in the sclera (sclerotomy) for outflow of aqueous.
A partial thickness scleral flap covers the opening and
the conjunctiva overlies the flap. Intraocular pressure can
be significantly lowered with this. Success rate in adults
is usually around 70% - 80%. In children, long term success rate is lower being around 50% [38] due to more
aggressive wound healing response in children, which
scars down sclerotomy or scleral flap. Children are also
more prone to complications such as infections due to
eye rubbing.

8.3. Management of Refractory Pediatric

Glaucomas (Figure 1)
When the IOP is not controlled after the first surgery, the
surgical options are filtration surgery with anti-fibrosis
drugs; glaucoma drainage implants or cyclodestructive
procedures.
8.3.1. Trabeculectomy with Anti-Fibrotic Agents
Mitomycin-C and 5-Florouracil are the two most commonly used anti-metabolites in glaucoma surgery. Although each of these medications decrease the scarring of
blebs, they also create more potential for complications
such as infection (endopthalmitis) [39-42].
8.3.2. Aqueous Drainage Implants
Implant surgery is an important treatment alternative for
PCG patients who are poor candidates for angle incision
therapy and trabeculectomy or who have proven to be
refractory to these procedures. Glaucoma drainage implants may be characterized as non-restrictive devices
such as Molteno and Baerveldt implants or valved such
as krupin implants or Ahmed glaucoma valve. Reported
complications of implants include hypotony with shallow
anterior chamber, choroidal detachments, tube cornea
touch, obstructed tube or plate, endophthalmitis and retinal detachment.
The surgical procedure is same for all implants [43].
The superior temporal quadrant is the preferred site. Following periotomy, the implant device is placed with its
anterior edge approximately 8mm from limbus. The tube
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Childhood Glaucoma: An Overview

is shortened and beveled and an incision is made into the

anterior chamber to allow entry of tube parallel to iris.
The tube is then protected with autologous sclera or
pericardium and limbal peritomy is closed.
8.3.3. Cyclodestructive Procedures
These are selectively used for PCG which has proven
refractory to medical therapy and to conventional surgical procedures to improve aqueous outflow and work by
decreasing aqueous production. The required ciliary
epithelial ablation is produced by trans-scleral cyclocryotherapy or by endoscopic diode laser cyclophotocoagulation. The ocular indications include a blind painful eye, a
blind eye with high pressure, rapidly deteriorating cornea
refractory to all treatment alternatives and, an eye with
anatomic defect which preclude other glaucoma procedures.

8.4. Low Vision Rehabilitation

Regrettably, children with congenital glaucoma may end
up with low vision despite treatment. Various low vision
aids such as magnifiers, binoculars, telescopes are available for such patients to improve their quality of life.
Some non-optical devices such as close circuit televisions, large print books exist for their help. Such patients
should receive special care and vocational training from
trained professionals, working specifically for visually
impaired patients, in order to help them develop their
abilities to maximum potential [44].

9. Conclusion
The main goal in managing primary congenital glaucoma
is early diagnosis and therefore early surgical intervention. Following successful surgery, glasses should be
prescribed and care must be taken to manage amblyopia
for optimal visual rehabilitation.

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